Infantile intestinal leiomyosarcoma: surgical resection (without adjuvant therapy) for cure
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Published on: July 28, 2020
B B Simpson1, E M Reynolds, S H Kim
1Department of Pediatric Surgery, Massachusetts General Hospital, Boston 02114, USA.
Insights
Infantile intestinal leiomyosarcomas are rare, but infants with these tumors have a favorable prognosis. Complete surgical removal leads to excellent outcomes, even without further treatment.
Area of Science:
- Pediatric Oncology
- Surgical Pathology
Background:
- Leiomyosarcomas are rare in infants, with differing primary sites compared to adults.
- Adult prognostic indicators for leiomyosarcomas may not apply to pediatric cases.
Observation:
- A 7-week-old infant presented with intussusception and a cecal mass.
- The mass was diagnosed as an intermediate-grade leiomyosarcoma.
- The infant achieved disease-free survival for 3 years post-resection without adjuvant therapy.
Findings:
- Complete surgical excision is the primary curative treatment for infantile intestinal leiomyosarcomas.
- Infantile leiomyosarcomas appear less aggressive than adult counterparts.
- Histological prognostic factors from adult leiomyosarcomas are not directly applicable to infants.
Implications:
- Early diagnosis and complete resection are crucial for favorable outcomes in infantile intestinal leiomyosarcomas.
- Adjuvant therapy may not be necessary for infants with completely resected leiomyosarcomas.
- Further research is needed to establish infant-specific prognostic factors for this rare malignancy.
Abstract:
A 7-week-old boy presented with a 6-week history of failure to thrive, acute intestinal obstruction, and an apparently irreducible intussusception (noted on contrast enema). He underwent abdominal exploration, during which a cecal mass was identified and resected. The mass proved to be a leiomyosarcoma. Histologically, it was an intermediate-grade malignancy with a predicted 5-year survival rate of 16% to 23% based on data from the adult experience. Three years after resection and without having received adjuvant therapy, he is healthy and free of disease. A review of the literature showed that in infants these tumors are predominantly colonic, compared with the predilection for small intestinal lesions found in the older pediatric and adult populations. Infantile intestinal leiomyosarcomata are rare malignancies that do well if complete surgical excision of the disease can be accomplished. The histological prognostic indicators proposed for intestinal leiomyosarcomas in the adult population cannot be extrapolated to infants because when they occur in infants, they appear to be less aggressive, and these patients do well without adjuvant therapy.
